Systemic Mast Cell Disease
Authors: RAYMOND D. MUTTER
Journal: Annals of Internal Medicine (1963)

Case Studies1 December 1963Systemic Mast Cell DiseaseRAYMOND D. MUTTER, M.D., MYRON TANNENBAUM, M.D., PH.D., JOHN E. ULTMANN, M.D., F.A.C.P.RAYMOND D. MUTTER, M.D.Search for more papers by this author, MYRON TANNENBAUM, M.D., PH.D.Search for more papers by this author, JOHN E. ULTMANN, M.D., F.A.C.P.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-59-6-887 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptUrticaria pigmentosa was first described in 1869 by Nettleship (1) and named by Sangster (2) in 1878. In this disease, mast cell infiltration is confined entirely to the skin. By 1923, 306 cases had been reported in the dermatologic literature (3), and at present over 600 cases have been recorded. In 1933, Touraine, Solente, and Renault (4) described urticaria pigmentosa with mast cells involving organs other than the skin. Since then, a total of 28 cases with systemic mast cell disease have been reported (4-30).The mast cell or tissue basophil was described, as well as named, by Paul Ehrlich...References1. NETTLESHIP E: Rare forms of urticaria. Brit. Med. J. 2: 323, 1869. MedlineGoogle Scholar2. SANGSTER A: An anomalous mottled rash, accompanied by pruritus, facticious urticaria and pigmentation, "urticaria pigmentosa." Trans. Clin. Soc. Lond. 11: 161, 1878. Google Scholar3. FINNERUD CW: Urticaria pigmentosa (nodular type) with a summary of the literature; report of a case. Arch. Derm. Syph. (Berlin) 8: 344, 1923. CrossrefGoogle Scholar4. TOURAINESOLENTERENAULT AGP: Urticaire pigmentaire avec reaction splenique et myelemique. Bull. Soc. Franc. Derm. Syph. 40: 1691, 1933. Google Scholar5. BERTELLOTTI L: Case report of urticaria pigmentosa with systemic involvement. G. Ital. Derm. Sif. 184: 698, 1943. Google Scholar6. ELLIS JM: Urticaria pigmentosa: report of a case with autopsy. Arch. Path (Chicago) 48: 426, 1949. MedlineGoogle Scholar7. BALBI E: Ricerche intorno alla patogenesi dell'urticaria pigmentosa. G. Ital. Derm. Sif. 190: 82, 1949. Google Scholar8. HISSARDMONOCOURIERJACQUET RLJ: Etude d'une nouvelle hematodermie: la mastocytose. Presse Med. 59: 1765, 1951. MedlineGoogle Scholar9. DEGOSLORTAT-JACOBHEWITTOSSIPOWSKI REJB: Reticulose a mastocytes, forme cutanee diffuse. Bull. Soc. Franc. Derm. Syph. 59: 247, 1952. MedlineGoogle Scholar10. BLUEFARBSALK SMMR: Urticaria pigmentosa with bone lesions, gastrointestinal symptoms and splenomegaly. Arch. Derm. Syph. (Berlin) 70: 376, 1954. Google Scholar11. REILLYSHINTANIGOODMAN EBJJR: Systemic mast cell disease with urticaria pigmentosa. Arch. Derm. (Chicago) 71: 561, 1955. Google Scholar12. BERLIN C: Urticaria pigmentosa as systemic disease. Ibid. 703. Google Scholar13. SAGHERLIBANUNGARSCHORR FEHS: Urticaria pigmentosa with bone involvement. J. Invest. Derm. 27: 355, 1956. CrossrefMedlineGoogle Scholar14. BRODEURGARDNER PLI: Urticaria pigmentosa as problem in diagnosis; report of two cases, one with systemic involvement. New Eng. J. Med. 254: 1165, 1956. CrossrefMedlineGoogle Scholar15. STARKVAN BUSKIRKDALY EFWJF: Radiologic and pathologic bone changes associated with urticaria pigmentosa; report of case. Arch. Path. (Chicago) 62: 143, 1956. MedlineGoogle Scholar16. ASBOE-HANSENKAALUND-JORGENSEN GO: Systemic mast cell disease involving skin, liver, bone marrow and blood associated with disseminated xanthomata. Acta Haemat. (Basel) 16: 273, 1956. CrossrefMedlineGoogle Scholar17. ASBOE-HANSEN G: Urticaria pigmentosa with generalized tissue mastocytosis and blood basophilia. Arch. Derm. (Chicago) 81: 198, 1960. CrossrefMedlineGoogle Scholar18. ASBOE-HANSEN G: Urticaria pigmentosa and generalized mastocytosis. Polymyxin B treatment. Arch. Derm. (Chicago) 83: 893, 1961. CrossrefMedlineGoogle Scholar19. RIDERSTEINABBUHL TLAAJW: Generalized mast cell disease and urticaria pigmentosa; report of case. Pediatrics 19: 1023, 1957. MedlineGoogle Scholar20. WATERSLACSON WJPS: Mast cell leukemia presenting as urticaria pigmentosa. Ibid. 1033. Google Scholar21. ZAKCOVEYSNODGRASS FGJAJJ: Osseous lesions in urticaria pigmentosa. New Eng. J. Med. 256: 56, 1957. CrossrefMedlineGoogle Scholar22. EFRATIKLAJMANSPITZ PAH: Mast cell leukemia—malignant mastocytosis with leukemia-like manifestations. Blood 12: 869, 1957. CrossrefMedlineGoogle Scholar23. FRIEDMANWILLFREIMANBRAUNSTEIN BIJJDGH: Tissue mast cell leukemia. Blood 13: 70, 1958. CrossrefMedlineGoogle Scholar24. ENDECHERNISS NEI: Splenic mastocytosis. Ibid. 631. Google Scholar25. HARVARDSCOTT CWRB: Urticaria pigmentosa with visceral and skeletal lesions. Quart. J. Med. 28: 459, 1959. MedlineGoogle Scholar26. BRINKMAN E: Mast cell reticulosis (tissue basophilia) with histamine conditioned flush and transition into tissue basophile leukemia. 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Reprinted in Mast Cells and Basophils, ed. by PADAWER, J. Ann. NY Acad Sci. 103: fol. page 492, 1963. Google Scholar33. JORPESHOLMGRENWILANDER EHO: Heparin in vessel walls and in the eyes. Z. Mikr. Anat. Forsch. 42: 279, 1937. Google Scholar34. ASBOE-HANSEN G: Origin of synovial mucin; Ehrlich's mast cell-secretory element of connective tissue. Ann. Rheum. Dis. 9: 149, 1950. CrossrefMedlineGoogle Scholar35. SCHILLERDORFMAN SA: Isolation of heparin from mast cells of the normal rat. Biochim. Biophys. Acta 31: 278, 1959. CrossrefMedlineGoogle Scholar36. RILEYWEST JFGB: Presence of histamine in tissue mast cells. J. Physiol. (London) 120: 528, 1953. CrossrefGoogle Scholar37. BENDITTWONGARASEROEPER EPRLME: 5-hydroxytryptamine in mast cells. Proc. Soc. Exp. Biol. Med. 90: 303, 1955. CrossrefMedlineGoogle Scholar38. LAGUNOFFBENDITT DEP: Proteolytic enzymes of mast cells. Ann. NY Acad. Sci. 103: 185, 1963. CrossrefMedlineGoogle Scholar39. 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A study of nine cases with special reference to the excretion of histamine in urine. Acta Med. Scand. 163: 223, 1959. CrossrefMedlineGoogle Scholar46. ONOZOMPETTIHAGENFURTH SLPJ: Relation of mastocytoma to mast cell leukemia, and of heparin, histamine and serotonin to mast cells. Blood 14: 770, 1959. CrossrefMedlineGoogle Scholar47. SAGHERSCHORR FS: Bone lesions in urticaria pigmentosa; report of central registry on skeletal x-ray survey. J. Invest. Derm. 26: 431, 1956. CrossrefMedlineGoogle Scholar48. POPPELGRUBERSILBERHOLDERCHRISTMAN MHWFRAKRO: Roentgen manifestations of urticaria pigmentosa (mastocytosis). Amer. J. Roentgen. 82: 239, 1959. Google Scholar49. URNÄS B: Mechanism of histamine release in mast cells. Ann. NY Acad. Sci. 103: 278, 1963. MedlineGoogle Scholar50. PADAWER J: Quantitative studies with mast cells. Ann. NY Acad. Sci. 103: 87, 1963. CrossrefGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: New York, N. Y.From the Departments of Medicine and Pathology, College of Physicians & Surgeons, Columbia University, and the Medical and Pathology Services, Francis Delafield Hospital, New York, N. Y.This work was supported in part by the National Cancer Institute grant CY-2332, National Institutes of Health, United States Public Health Service, Bethesda, Maryland; the Health Research Council of the City of New York; and the Anne Winton Memorial Fund, New York, N. Y.Dr. Ultmann is a Career Scientist, grant 1-109, Health Research Council of the City of New York.Requests for reprints should be addressed to John E. Ultmann, M.D., Institute of Cancer Research, College of Physicians & Surgeons of Columbia University, 630 West 168th St., New York 32, N. Y. 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